Treatment plans for intractable pneumothorax and pleuritis accompanied with Mycobacterium avium complex infection have not been well defined. We describe a 64-year-old female patient whose multidrug therapy treatment for pulmonary infection with M. avium complex had continued for 3 years. Six months after cessation of this chemotherapy, a chest radiograph showed pneumothorax and pleural effusion with extensive parenchymal infiltration in the right hemithorax. The patient's chest CT scan and pleural fluid analysis showed pneumothorax and pleuritis with M. avium complex infection. Although chemotherapy and chest space drainage decreased pleural effusion, pulmonary air leakage and pneumothorax remained. We performed fiberoptic bronchoscopy with endoscopic bronchial occlusion two times, and this stopped the air leakage and made the right lung fully expanded. We successfully removed the chest tube after using chemical pleurodesis, and the patient was discharged. This report suggests that bronchial occlusion can be highly effective in treating intractable pneumothorax and pleuritis accompanied with M. avium complex infection.